ABSTRACT

Amyloidosis is a protein misfolding disorder leading to the accumulation in tissues of protein aggregates in a fibrillar form having a cross-β sheet conformation. This chapter shows that anemic patients with AA amyloidosis have significantly elevated circulating levels of interleukin (IL)-1β and of IL-18 as compared with matched nonanemic amyloid patients, and that the occurrence of anemia in amyloidosis is associated with allele 2 (T) of the IL-1 β -511 promoter gene. The pathogenesis of anemia in reactive AA amyloidosis is complex. The amyloid patients in this series had either normal or only mild renal dysfunction which argues against the possibility that renal anemia to any appreciable degree contributed to the anemia in these patients. The chapter concludes that the occurrence of anemia in patients with reactive AA amyloidosisis associated with allele 2 (T) of the Il-1β -511 promoter gene and elevated levels of IL-1β and IL-18.